免疫原
The antiserum was produced against synthesized peptide derived from human GK. AA range:461-510
特异性
GK1 Monoclonal Antibody detects endogenous levels of GK1 protein.
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺(Immunogen)
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
背景(Background)
The protein encoded by this gene belongs to the FGGY kinase family. This protein is a key enzyme in the regulation of glycerol uptake and metabolism. It catalyzes the phosphorylation of glycerol by ATP, yielding ADP and glycerol-3-phosphate. Mutations in this gene are associated with glycerol kinase deficiency (GKD). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2011],
功能
catalytic activity:ATP + glycerol = ADP + sn-glycerol 3-phosphate.,caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:Defects in GK are the cause of GK deficiency (GKD) [MIM:307030]. This disease can be either symptomatic with episodic metabolic and CNS decompensation or asymptomatic with hyperglycerolemia and hyperglyceroluria only.,function:Key enzyme in the regulation of glycerol uptake and metabolism.,pathway:Polyol metabolism; glycerol degradation via glycerol kinase pathway; sn-glycerol 3-phosphate from glycerol: step 1/1.,similarity:Belongs to the FGGY kinase family.,subcellular location:In sperm and fetal tissues, the majority of the enzyme is bound to mitochondria, but in adult tissues, such as liver found in the cytoplasm.,tissue specificity:Highly expressed in the liver, kidney and testis
其他名称
GK; Glycerol kinase; GK; Glycerokinase; ATP:glycerol 3-phosphotransferase
Fields
>>Glycerolipid metabolism;>>Metabolic pathways;>>PPAR signaling pathway
细胞定位
Mitochondrion outer membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm. In sperm and fetal tissues, the majority of the enzyme is bound to mitochondria, but in adult tissues, such as liver found in the cytoplasm.
组织表达
Highly expressed in the liver, kidney and testis. Isoform 2 and isoform 3 are expressed specifically in testis and fetal liver, but not in the adult liver.